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GROWTH AND FEEDING
M.Cristina Digilio
Bambino Gesù Pediatric Hospital - Rome
16th Annual International MeetingThe Velo-Cardio-Facial Syndrome Educational Foundation, Inc.
July 3 – 5, Roma
Several different causes
Feeding difficulties(40% of the cases)
hospitalizationcongenitalheart defect cleft palate
dysmotility of thedigestive tract …
Growth and feeding
Prenatal manifestation POLYHYDRAMNIOS
• Polyhydramnios is detectable in 10 % of the patients with del22
• The onset is generally in the beginning of the second trimester
• Decreased swallowing contributes to polyhydramnios
• In many patients presenting with severe polyhydramnios, major feeding problems are observed in the first year of life
Vantrappen et al., Genet Couns 1999
Growth and feeding
Feeding difficulties
Birth weight
• Normal weight (between 3rd and 97th centile): 84 %• Weight < 50th centile: 72 %• Weight < 3° centile: 16 %
• Mean birthweight (males – 38th week): 2960 g • Mean birthweight (females – 38th week): 2760 g
Growth
Ryan et al., J Med Genet 1997
Growth and feeding
Feeding difficulties
Preferred spoon feeding Difficulties in cup drinkingPreferred consecutive swallows without taking a breath
Growth and feeding
VomitingNasal regurgitationCoughingTendency to choking
Feeding difficulties
Difficulties in chewing with solidsAccepting only a selected few foods
Growth and feeding
VomitingNasal regurgitationCoughingTendency to choking
Preferred spoon feeding Difficulties in cup drinkingPreferred consecutive swallows without taking a breath
• Anatomic defects: cleft palate vascular anomalies (right aortic arch, aberrant subclavian artery) congenital heart defect laryngomalacia
• Functional defects : pharyngeal hypotonia glossoptosis gastro-esophageal refluxes dismotility and hypotonia of the digestive tract infections of the respiratory tract
Feeding difficulties
CAUSES
Growth and feeding
Gastrointestinal malformations:
• esophageal atresia• intestinal atresia
• Hirschprung disease• imperforate anus
Feeding difficulties
CAUSES
Growth and feeding
• Endoscopic evaluation
• Barium swallow
• Angio MRI of the aortic vessels
Feeding difficulties
EVALUATION
Growth and feeding
• Feeding techniques (frequent feeding with small quantities, specific nipples, upright position)
• Medical treatment of gastro-esophageal reflux
• Gavage feeding, gastrostomy
• Surgical correction of malformations
Feeding difficulties
TREATMENT
Growth and feeding
Weight
• Weight deficiency in the first years of age• Weight normalization in the following years• Predisposition to develop obesity in adolescence
Growth and feeding
Causes of weight deficiency
• CONGENITAL HEART DEFECT not significant (p = 0.690)
• CLEFT PALATE not significant (p = 0.756)
• FEEDING DIFFICULTIES significant (p 0.004)
Cause Comparison
Growth and feeding
Height
• Short stature (<3rd centile) in 14 % of the cases• The patients with short stature were all < 10 years old• Normal height in adolescence
Growth and feeding
Bone age
• 1-4 years: delay in bone age, corresponding to – 4/6 months
• 5-10 years: delay in bone age, corresponding to – 4/6 months
• 11-16 years: bone age corresponding to chronological age
Growth and feeding
Celiac disease
• The prevalence of celiac disease (CD) in patients with Del22 (2%) is not so high as in other types of genetic syndromes
• Screening for CD is indicated only in subjects presenting with persistent gastrointestinal symptoms or significant growth abnormalities
Growth and feeding
Growth: Periodical evaluations
• Evaluation of growth parameters (weight and length/height)
• Bone age every 2 years
• IGF1 blood dosage and pharmacological stimulation of growth hormone (GH) response in patients with height below the 3rd centile
• Annual evaluation of blood thyroid hormones (TSH, FT3, FT4) and, after 10 years of age, dosage of antiperossidasis antibodies (AbTPO)
Growth and feeding